Tenosynovial Giant Cell Tumour - Nodular
Synonyms: Localised TGCT, giant cell tumour of tendon sheath, nodular tenosynovitis, PVNS
Most common tumour of tendon sheath - palmar surface of digits is classic location
Quick Facts
Behaviour
Benign
Category
Soft tissue
Synonyms
- Localised TGCT
- giant cell tumour of tendon sheath
- nodular tenosynovitis
- PVNS
Category
Soft tissue
Behaviour
Benign
Gender
Female
Tissue of Origin
Synovial/tenosynovial tissue
Epidemiology
- Most common tumour of tendon sheath
- Peak incidence in 3rd–5th decades
- CSF1 overexpression drives tumour proliferation
Clinical Features
- Painless firm nodule on palmar surface of finger or hand
- Slowly growing, may cause pressure erosion of adjacent Bone
- Non-tender, lobulated, well-defined
- Restricted tendon gliding
Location
- Fingers (most common - palmar surface)
- Hand and wrist
- Ankle and foot
- Knee (rarer - localised form)
Imaging
- Well-defined lobulated hypointense mass on MRI (haemosiderin)
- Bone erosion on X-ray in 10%
- Low signal on both T1 and T2 (haemosiderin) - characteristic
- Ultrasound: solid hypoechoic mass adjacent to tendon
Pathology
- Synovial-type cells, osteoclast-type giant cells, foam cells, and haemosiderin
- CSF1 overexpression - drives tumour proliferation and macrophage recruitment
- Lobulated with Fibrous septae
- CSF1R mutation or translocation in a subset of cells
Genetics
- CSF1-COL6A3 translocation (2p13; 1p13) in subset of neoplastic cells
- CSF1 overexpression recruits non-neoplastic macrophages (tumour microenvironment)
- Clonal neoplasm despite reactive appearance
Treatment
- Surgical excision - marginal excision curative in most
- Complete excision of all lobules reduces recurrence risk
- Pexidartinib (CSF1R inhibitor): FDA-approved for symptomatic TGCT not amenable to surgery
Prognosis
- Local recurrence 10–20% after marginal excision
- No Malignant potential for localised type
- Pexidartinib achieves durable responses in unresectable cases
Key Points
- Most common tumour of tendon sheath - palmar surface of digits is classic location
- Low signal on T1 and T2 MRI due to haemosiderin is characteristic
- Pexidartinib (CSF1R inhibitor) is FDA-approved for symptomatic unresectable TGCT
- Complete excision of all lobules is key to reducing recurrence
Workup - Blood Tests
No routine blood tests required
Workup - Local Imaging
- Ultrasound or MRI tendon sheath - well-defined hypoechoic/hypointense nodule; haemosiderin (Low T1/T2)
- Plain radiograph - assess for Bone erosion
Workup - Biopsy
Biopsy rarely required if imaging characteristic
Workup - Staging
None required
Follow-up Summary
- 1
Benign soft tissue tumour (localised TGCT) - Follow-up to detect local recurrence
- 2
Post-op visit at 6 weeks; USS or MRI of tendon sheath at 3 months as new baseline
- 3
Year 1
Single review at 6 months - clinical exam + USS
- 4
Year 2
Annual review; USS if symptomatic
- 5
Discharge at 2 years if asymptomatic and no clinical recurrence
- 6
Recurrence (10–20%)
re-excision; if multiple recurrences consider pexidartinib referral
- 7
No systemic metastatic surveillance required
Medical disclaimer
The content on Sarcopedia is for educational and informational purposes only and does not constitute medical advice. It is not intended to be a substitute for professional medical diagnosis or treatment. Always consult with a qualified physician regarding any health concerns or before starting any new treatment. Reliance on any information provided on this site is solely at your own risk.